Hearing Screening Training Manual - Minnesota Department of Health

6. Physical findings, such as white forelock, that are associated with a syndrome known to
include a sensorineural or permanent conductive hearing loss.
7. Syndromes associated with hearing loss or progressive or late-onset hearing loss, such as
neurofibromatosis, osteopetrosis, and Usher syndrome; other frequently identified syndromes
include Waardenburg, Alport, Pendred, and Jervell and Lange-Nielson.
8. Neurodegenerative disorders, such as Hunter syndrome, or sensory motor neuropathies,
such as Friedreich ataxia and Charcot-Marie-Tooth syndrome.
There are many syndromes associated with hearing loss that include observable physical anomalies of
the head, neck, and ears which frequently result in hearing loss (e.g., Down syndrome). Another
example is Neurofibromatosis Type II (NF2), an inherited tumor syndrome. Some syndromes are not
evident at birth.
9. Culture-positive postnatal infections associated with sensorineural hearing loss, including
confirmed bacterial and viral (especially herpes viruses and varicella) meningitis.
Type B, Hemophilus Influenza, carries the greatest incidence of hearing loss. Bacterial
meningitis is the leading cause of acquired deafness in infants and childhood, ranging in
incidence from 5-30%. Most occurrences result in severe, bilateral, symmetrical, sensorineural
hearing loss; 30% have profound loss. The age at which meningitis occurs significantly affects
rehabilitative needs; the younger the child, the greater the impact on speech and language
acquisition.
10. Head trauma, especially basal skull/temporal bone fractures that requires hospitalization.
Head trauma (e.g., a skull fracture) may affect hearing due to potential damage to either the
cochlea or middle ear, resulting in sensorineural or conductive hearing loss. Sensorineural loss
may occur due to damage or obliteration of the temporal bone housing the inner ear. Conductive
hearing loss occurs as a result of perforation of the tympanic membrane, bleeding, or disruption of
the ossicular chain.
11. Chemotherapy.
“Chemotherapy” refers to a number of drugs which can be “ototoxic”
Ototoxicity is damage to the ear (oto-), specifically the cochlea or auditory nerve and sometimes
the vestibular system, by a toxin. It is commonly medication-induced; ototoxic drugs include
antibiotics such as the aminoglycoside gentamicin, loop diuretics such as furosemide, and
platinum-based chemotherapy agents such as cisplatin. It can result in sensorineural hearing loss,
dysequilibrium, or both. Either may be reversible and temporary, or irreversible and permanent.
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